Rare immunodeficiency provides new insight into immune system function

Scientists now have a better understanding of the complex events that contribute to the pathology of an inherited immunodeficiency called X-linked lymphoproliferative disease (XLP). Two separate research studies investigating this rare disorder provide critical new information about the regulation of T helper cell cytokine secretion, a process that is absolutely critical for appropriate immune responses to infectious agents. The research is published in the November issue of Immunity.

XLP is a rare, inherited immunodeficiency that is characterized by an abnormal and extreme sensitivity to the Epstein-Barr virus (EBV). XLP patients exposed to EBV have a severe and often fatal massive proliferation of abnormal T lymphocytes. Those individuals who survive the EBV infection have a significant risk of developing lymphomas. XLP is caused by mutation of a gene expressed in T cells that encodes a protein called SAP. Although the precise dysregulation of T cells that underlies XLP is unclear, research using genetically engineered mice that lack SAP demonstrated that T helper cells in these animals exhibit reduced cytokine production.

Dr. Pamela L. Schwartzberg from the National Human Genome Research Institute and colleagues used SAP-deficient T cells to investigate specific aspects of the T cell signaling cascade. Some components of the signaling cascade used by activated T cells are unperturbed in the mutant T cells whereas other aspects are impaired. Dr. Schwartzberg's group dissects specific T cell signaling molecules, such as Fyn, that are linked to reduced cytokine production in the SAP mutants. "Our data argue that a SAP/Fyn pathway may be required for the efficient recruitment of PKC-q and Bcl-10, as well as proper patterns of activation of NF-kB, and suggest a potentially novel pathway of T helper cell cytokine activation," offers Dr. Schwartzberg.

A separate study led by Dr. Andre Veillette from the Institut de recherches cliniques de Montrea

Contact: Heidi Hardman
Cell Press

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